The switch from fetal to adult hemoglobin relies on repression or silencing of the upstream γ-globin gene, but identification of the transcriptional repressors that bind to the sites at which a cluster of naturally occurring variants associated with HPFH (hereditary persistence of fetal hemoglobin) are found has been elusive. A new study provides mechanistic evidence for the direct binding of BCL11A and ZBTB7A, two previously identified γ-globin gene repressors.
References
Stamatoyannopoulos, G. Exp. Hematol. 33, 259–271 (2005).
Thein, S. L., Menzel, S., Lathrop, M. & Garner, C. Hum. Mol. Genet. 18, R216–R223 (2009).
Giardine, B. et al. Nat. Genet. 43, 295–301 (2011).
Xu, J. et al. Genes Dev. 24, 783–798 (2010).
Jawaid, K., Wahlberg, K., Thein, S. L. & Best, S. Blood Cells Mol. Dis. 45, 140–146 (2010).
Martyn, G. E. et al. Nat. Genet. http://dx.doi.org/10.1038/s41588-018-0085-0 (2018).
Menzel, S. et al. Nat. Genet. 39, 1197–1199 (2007).
Uda, M. et al. Proc. Natl Acad. Sci. USA 105, 1620–1625 (2008).
Bauer, D. E. et al. Science 342, 253–257 (2013).
Xu, J. et al. Science 334, 993–996 (2011).
Canver, M. C. et al. Nature 527, 192–197 (2015).
Maeda, T. et al. Nature 433, 278–285 (2005).
Masuda, T. et al. Science 351, 285–289 (2016).
Hoban, M. D., Orkin, S. H. & Bauer, D. E. Blood 127, 839–848 (2016).
Traxler, E. A. et al. Nat. Med. 22, 987–990 (2016).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no competing interests.
Rights and permissions
About this article
Cite this article
Wang, X., Thein, S. Switching from fetal to adult hemoglobin. Nat Genet 50, 478–480 (2018). https://doi.org/10.1038/s41588-018-0094-z
Published:
Issue Date:
DOI: https://doi.org/10.1038/s41588-018-0094-z
- Springer Nature America, Inc.
This article is cited by
-
Therapeutic adenine base editing of human hematopoietic stem cells
Nature Communications (2023)
-
CRISPR/Cas-based gene editing in therapeutic strategies for beta-thalassemia
Human Genetics (2023)
-
Genetic Modifiers of Fetal Haemoglobin in Sickle Cell Disease
Molecular Diagnosis & Therapy (2019)